title: Melanotic neuroectodermal tumor of infancy disseminated by a ventriculoperitoneal shunt and diagnosed from the inguinal sac.
abstract: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare congenital neoplasm that originates from the neural crest cells, which give rise to the melanocytes of the skin and leptomeninges. We report a case of MNTI with neurocutaneous melanosis of a 28-month-old girl. She was born with hydrocephalus and several large congenital giant nevi. There were no findings except for hydrocephalus, after a ventriculoperitoneal (VP) shunt operation performed when she was 6 months old. She was operated on for a growing inguinal mass at 8 months. The specimen from the inguinal sac was positive for HMB45, vimentin, chromogranin, and neuron-specific enolase. Brain magnetic resonance imaging showed an extensive enhancing extra-axial mass with high signal intensity, along the cerebral spinal fluid space. We report a rare case of MNTI, diagnosed from an inguinal hernia sac, with a disseminated clinical manifestation.
authors.affiliation: Departments of *Pediatrics †General Surgery, College of Medicine, The Catholic University of Korea, Seoul St. Mary's Hospital, Seocho-gu ‡Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: In Hyuk
authors.initials: IH
authors.lastname: Yoo
authors.forename: Sook Kyung
authors.initials: SK
authors.lastname: Yum
authors.forename: Se-Jeong
authors.initials: SJ
authors.lastname: Oh
authors.forename: Kyoung-Mee
authors.initials: KM
authors.lastname: Kim
authors.forename: Dae Chul
authors.initials: DC
authors.lastname: Jeong
mesh_terms: D001932:Brain Neoplasms / Q000473:pathology* / Q000601:surgery*; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000601:surgery*; D007264:Inguinal Canal / Q000473:pathology; D009360:Neoplastic Cells, Circulating; D017600:Neuroectodermal Tumor, Melanotic / Q000473:pathology* / Q000601:surgery*; D009506:Nevus / Q000473:pathology; D017287:Ventriculoperitoneal Shunt / Q000009:adverse effects*